Cushing's disease: pathobiology, diagnosis, and management

J Neurosurg. 2017 Feb;126(2):404-417. doi: 10.3171/2016.1.JNS152119. Epub 2016 Apr 22.


Cushing's disease (CD) is the result of excess secretion of adrenocorticotropic hormone (ACTH) by a benign monoclonal pituitary adenoma. The excessive secretion of ACTH stimulates secretion of cortisol by the adrenal glands, resulting in supraphysiological levels of circulating cortisol. The pathophysiological levels of cortisol are associated with hypertension, diabetes, obesity, and early death. Successful resection of the CD-associated ACTH-secreting pituitary adenoma is the treatment of choice and results in immediate biochemical remission with preservation of pituitary function. Accurate and early identification of CD is critical for effective surgical management and optimal prognosis. The authors review the current pathophysiological principles, diagnostic methods, and management of CD.

Keywords: ATCH = adrenocorticotropic hormone; CD = Cushing's disease; CRH = corticotropin-releasing hormone; CRH-R1 = CRH receptor-1; CS = Cushing's syndrome; Cushing's disease; IPSS = inferior petrosal sinus sampling; SPGR = spoiled gradient recalled; SRS = stereotactic radiosurgery; diagnosis; oncology; surgery; treatment.

Publication types

  • Review

MeSH terms

  • Humans
  • Pituitary ACTH Hypersecretion* / diagnosis
  • Pituitary ACTH Hypersecretion* / physiopathology
  • Pituitary ACTH Hypersecretion* / therapy